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迟发性重症肌无力的胸腺切除术与抗肌肉自身抗体

Thymectomy and anti-muscle autoantibodies in late-onset myasthenia gravis.

作者信息

Romi F, Gilhus N E, Varhaug J E, Myking A, Skeie G O, Aarli J A

机构信息

Department of Neurology, Haukeland University Hospital, Bergen, Norway.

出版信息

Eur J Neurol. 2002 Jan;9(1):55-61. doi: 10.1046/j.1468-1331.2002.00352.x.

DOI:10.1046/j.1468-1331.2002.00352.x
PMID:11784377
Abstract

Thymectomy is still widely carried out in myasthenia gravis (MG) patients, but its role, especially in late-onset MG patients, is not established. These patients are immunologically heterogeneous, some with thymoma-like and others with early onset-like features. We evaluated whether any therapeutic effects of thymectomy correlate with the presence of non-acetylcholine receptor (AChR) muscle antibodies. The severity of MG, and titin and ryanodine receptor (RyR) antibodies, were assessed yearly starting from MG onset in 21 thymectomized and 22 non-thymectomized AChR antibody positive late-onset MG patients, who were followed for 2, 3 and 5 years. Clinical or pharmacological remission were seen in six of 11 titin antibody negative but none of the 10 titin antibody positive thymectomized patients, however, the non-thymectomized cases showed an opposite trend. The three MG-related deaths were all in patients with titin antibodies. There was no significant difference in MG severity between thymectomized and non-thymectomized patients; 2 years after MG onset, both groups were significantly improved. This study showed no dramatic benefit from thymectomy in late-onset MG in general. Any limited improvement appeared less likely in cases with titin and/or RyR antibodies.

摘要

胸腺切除术仍在重症肌无力(MG)患者中广泛开展,但其作用,尤其是在晚发型MG患者中的作用尚未明确。这些患者在免疫学上具有异质性,一些具有胸腺瘤样特征,另一些具有早发型样特征。我们评估了胸腺切除术的任何治疗效果是否与非乙酰胆碱受体(AChR)肌肉抗体的存在相关。从MG发病开始,每年对21例接受胸腺切除术和22例未接受胸腺切除术的AChR抗体阳性晚发型MG患者的MG严重程度、肌联蛋白和兰尼碱受体(RyR)抗体进行评估,随访2年、3年和5年。11例肌联蛋白抗体阴性的胸腺切除患者中有6例出现临床或药理学缓解,但10例肌联蛋白抗体阳性的胸腺切除患者均未出现缓解,然而,未接受胸腺切除术的患者呈现相反趋势。3例与MG相关的死亡均发生在有肌联蛋白抗体的患者中。胸腺切除患者和未接受胸腺切除术患者的MG严重程度无显著差异;MG发病2年后,两组均有显著改善。这项研究表明,总体而言,胸腺切除术对晚发型MG没有显著益处。在有肌联蛋白和/或RyR抗体的病例中,任何有限的改善似乎都不太可能出现。

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引用本文的文献

1
Can Non-Thymomatous Late-Onset Myasthenia Gravis Benefit From Thymectomy? A Systematic Review and Meta-Analysis.非胸腺瘤性迟发性重症肌无力能从胸腺切除术中获益吗?一项系统评价和Meta分析
Eur J Neurol. 2025 Mar;32(3):e70048. doi: 10.1111/ene.70048.
2
Effects of thymectomy on late-onset non-thymomatous myasthenia gravis: systematic review and meta-analysis.胸腺瘤切除术后对迟发性非胸腺瘤型重症肌无力的影响:系统评价和荟萃分析。
Orphanet J Rare Dis. 2021 May 20;16(1):232. doi: 10.1186/s13023-021-01860-y.
3
Effect of thymectomy in elderly patients with non-thymomatous generalized myasthenia gravis.
胸腺切除术对非胸腺瘤性全身性重症肌无力老年患者的影响。
J Neurol. 2019 Apr;266(4):960-968. doi: 10.1007/s00415-019-09222-2. Epub 2019 Feb 6.
4
Muscle autoantibodies in myasthenia gravis: beyond diagnosis?重症肌无力中的肌肉自身抗体:超越诊断?
Expert Rev Clin Immunol. 2012 Jul;8(5):427-38. doi: 10.1586/eci.12.34.